Quality of life in adolescents with chronic kidney disease who initiate haemodialysis treatment.

BACKGROUND: To describe the quality of life of adolescents initiating haemodialysis, to determine the factors associated with quality of life, and to assess coping strategies and their impact on quality of life. METHODS: All adolescents initiating haemodialysis between September 2013 and July 2015 in French paediatric haemodialysis centres were included. Quality of life data were collected using the "Vécu et Santé Perçue de l'Adolescent et l'Enfant" questionnaire, and coping data were collected using the Kidcope questionnaire. Adolescent's quality of life was compared with age- and sex-matched French control. RESULTS: Thirty-two adolescents were included. Their mean age was 13.9 ± 2.0 years. The quality of life score was lowest in leisure activities and highest in relationships with medical staff. Compared with the French control, index, energy-vitality, relationships with friends, leisure activities and physical well-being scores were significantly lower in haemodialysis population. In multivariate analyses, active coping was positively associated with quality of life and especially with energy-vitality, relationships with parents and teachers, and school performance. In contrast, avoidant and negative coping were negatively associated with energy-vitality, psychological well-being and body image for avoidant coping, and body image and relationships with medical staff for negative coping. CONCLUSIONS: The quality of life of haemodialysis adolescents, and mainly the dimensions of leisure activities, physical well-being, relationships with friends and energy-vitality, were significantly altered compared to that of the French population. The impact of coping strategies on quality of life seems to be important. Given the importance of quality of life and coping strategies in adolescents with chronic disease, health care professionals should integrate these aspects into care management.

[Peritoneal equilibration test: Conventional versus adapted. Preliminary study]. / Tests d'équilibration péritonéaux : conventionnel versus adapté. Étude de faisabilité.

Conventional automated peritoneal dialysis (APD) is prescribed as a repetition of cycles with the same dwell time and the same fill volume. Water and sodium balance remains a common problem among patients on peritoneal dialysis. More recently, adapted automated peritoneal dialysis was described, as a combination of short dwells with a low volume, in order to enhance ultrafiltration, followed by long dwells with a large fill volume to favor solute removal. We performed a preliminary crossover study on 4 patients. The total amount of dialysate was the same, i.e. 2L/m2 as well as the total duration of the test, i.e. 150 minutes. The conventional test was made with two identical cycles, each cycle had a fill volume of 1L/m2 and a duration of 75 minutes, while the adapted test was performed with one short cycle, i.e. 30 minutes with a low fill volume, i.e. 0.6L/m2, followed by a long cycle, i.e. 120 minutes, with a large fill volume, i.e. 1.4L/m2. Sodium extraction was improved by 29.3mmol/m2 (169%) in the adapted test in comparison to the conventional test. Ultrafiltration was enhanced by 159mL/m2 (128%) in the adapted test compared to the conventional one. Glucose absorption was decreased by 35% in the adapted test in comparison to the conventional test and osmotic conductance was also improved. In conclusion, adapted dialysis may allow for a better volume and sodium balance, since we observed an improvement in sodium extraction and ultrafiltration. This pre-study authorizes an improvement of the European Pediatric Study's protocol on Adapted APD, already started and which will continue in the next months.

Why does three times per week hemodialysis provide inadequate dialysis for children?

The duration of chronic conventional dialysis is a risk factor in children, both in terms of growth retardation and cardiovascular morbidity and mortality. Therefore, we need to develop alternative strategies, such as preemptive kidney transplantation and/or more intensive dialysis prescription. Indeed, conventional hemodialysis could be improved in all children by the use of high permeable membrane and ultrapure dialysis fluids (having very low endotoxin levels); by the addition of a convective dialysis dose to the urea diffusion dialysis dose (Kt/Vurea), i.e., hemodiafiltration; moreover, by the preservation of cardiovascular morphology and function (optimized blood pressure control); and also by the prescription of more frequent/longer dialysis sessions.

Intensified daily dialysis: the best chronic dialysis option for children?

Children receiving chronic hemodialysis (HD) three times a week have many obstacles to overcome. Not only do they have to endure dietary restrictions, but they also need to take various medications on a daily basis, which contribute to anorexia. Children on such conventional dialysis programs often have poorly controlled blood pressure (which can lead to left ventricular hypertrophy and/or left ventricular dysfunction) and impaired statural growth. Therefore, the need for more frequent and/or intensive dialysis is recognized. Nevertheless despite limited center experience, daily dialysis is currently most often limited as a rescue treatment. When performed, daily intensified HD provides a modality for preserving cardiovascular health and promoting normal growth in children. Therefore, the time spent on chronic dialysis preserves their chances of the best possible outcome.

Daily on line haemodiafiltration promotes catch-up growth in children on chronic dialysis.

BACKGROUND: In children, growth can be used as a measurable parameter of adequate nutrition and dialysis dose. Despite daily administration of recombinant human growth hormone (rhGH), growth retardation remains a frequent problem in children on chronic dialysis. Therefore, we performed an observational prospective non-randomized study of children on in-centre daily on line haemodiafiltration (D-OL-HDF) dialysis with the aim of promoting growth. PATIENTS AND METHODS: Mean age at the start of the study was 8 years and 3 months, and all children had been receiving rhGH treatment for >12 months before enrolment. Mean follow-up time on D-OL-HDF was 20.5 +/- 8 months (range, 11-39 months). Renal residual function was either <3 mL/min/1.73 m(2) or anuric. Vascular access was a fistula (13/15) or a central venous catheter (2/15). Dialysis was delivered daily, six days a week in 3 hourly sessions (18 h/week), in a predilution OL-HDF mode, allowing a high convective volume (18 to 27 L/m(2) body surface area per session), Kt/V(urea) on line measured at least 1.4 per session. RESULTS: Mean growth velocity increased from 3.8 +/- 1.1 cm/year at inclusion to 14.3 +/- 3.8 cm/year during the first year of D-OL-HDF, resulting in a change in height standard deviation score (SDS) over the follow-up period from -1.5 +/- 0.3 SDS to +0.2 +/- 1.1 SDS. Increase in body mass was also noted without impaired control of blood pressure. Time-average deviation for urea (TAD(urea)) was low at 2.5 +/- 0.4 as was TAD(bicarbonate) due to the normal pre and post dialysis bicarbonate levels, respectively, 23.6 +/- 0.5 mmol/L and 26.6 +/- 0.5 mmol/L. The absence of any dietary restrictions permitted a mean protein diet intake (PDI) of 2.5 +/- 0.2 g/kg/day (PDI measured from a 3-day diet survey), contrasting with a mean normalized protein nitrogen appearance (nPNA) of 1.53 +/- 0.12 g/kg/day (nPNA calculated from urea dialytic kinetic). A low C-reactive protein was noted in 13/15 children, and mean beta(2) microglobulin was low, 15.3 +/- 0.3.3 mg/L. CONCLUSIONS: Daily OL-HDF promotes catch-up growth in children despite on chronic dialysis. This catch-up growth if continued, should allow the children to reach their mid-parental target height in the future. It could be speculated that the improved response to rhGH is the result of several combined factors conducting to less malnutrition and to less cachexia.

Importance of the curve shape for interpretation of blood volume monitor changes during haemodiafiltration.

In children, the prescribed ultrafiltration needed to achieve the fixed end session dry weight can induce hypotensive episodes. A variety of on-line devices based on the direct measurement of the hematocrit are available, but these devices nearly always only measure the quantitative variation in the blood volume as the means of identifying a hypotensive occurrence risk. In February 2002, our unit began using an on-line hematocrit measurement available even with infants' blood lines. Since January 2004, this blood volume monitor (BVM) has been used routinely in all dialysis sessions, and 2240 BVM data sets have been recorded and analysed during the last 4 years. Based on our analysis of these data sets, we have determined that, in addition to the described threshold points, which provide a quantitative analysis of the BVM, the qualitative analysis of the BVM, the so-called curve shape, is also of clinical importance. In 91% of the sessions analysed, a very similar "symptom-free" curve shape was noted that consisted of an initial decrease, followed by the BV reaching a "stable" plateau. Additional curve shapes were identified: one with no BV decrease, presumably indicating an overload risk state, and one with a continuous BV decrease, presumably indicating an hypovolemic risk state. In our experience, only 2% of the patients had relevant clinical symptoms that were not visible by BVM.

Unusual clinical severity of complement membrane cofactor protein-associated hemolytic-uremic syndrome and uniparental isodisomy.

Atypical hemolytic-uremic syndrome (aHUS; OMIM 235400) is genetically and clinically heterogeneous. Mutations in membrane cofactor protein (MCP; CD46), a widely expressed complement regulator, predispose to recurrent forms of the disease. Patients carrying MCP mutations have a favorable clinical outcome in comparison to those with factor H (CFH) or factor I (IF) mutations, which lead in most cases to end-stage renal failure. We identified 1 patient who presented at 1 year of age with a first episode of aHUS requiring dialysis therapy. After 2 recurrences of the disease, the patient developed end-stage renal failure. No mutation in the CFH and IF genes was found. A novel homozygous mutation (IVS10+2 T-->C) in the splice-donor of exon 10 encoding the transmembrane region of the MCP gene was associated with dramatically decreased cell-surface expression of MCP. Because the nucleotide substitution was inherited from the patient's father, but not her mother, a large deletion or uniparental disomy was suspected. Both karyotyping and cytogenetic analysis of chromosome 1q32 were performed, for which MCP maps showed no abnormalities. Subsequent genotype analysis using microsatellite markers spanning chromosome 1 showed that the affected child was homozygous for the entire series of markers tested and that all alleles originated from the father. Complete paternal uniparental isodisomy of chromosome 1 is a novel mechanism resulting in severe deficiency of MCP expression. The outcome of the disease reported here indicates that MCP mutation and complete paternal uniparental disomy of chromosome 1 could have an additive effect in determining the severity of the HUS phenotype.

Intensified and daily hemodialysis in children might improve statural growth.

In children conventional hemodialysis does not often improve growth. We determined linear growth in five children on in-center intensified and daily hemodialysis (IDd) regimen, with a mean age of 8 years 7 months at enrollment. Four of five were on growth hormone started for a median of 28.5 months before IDd. IDd was delivered 5 to 6 times weekly, for three hours each session. Mean follow up of IDd was 18.6 months. Dropout from IDd was kidney transplantation (n=4) or transfer to another center (n=1). IDd and free diet improved appetite, thereby protein intake, was above 2 g/kg/BW. Median weekly Kt/V(urea) was 9.1 (8.7 to 10.4). Predialysis phosphorus blood levels were higher at the start (2.04+/-0.34 mmol/L) than at end of IDd (1.39+/-0.41 mmol/L) without need for carbonate of calcium in four of five cases. During conventional dialysis ht SDS decreased from -0.8 to -1.44, which occurred predominantly before rhGH start. Conversion to IDd significantly increased growth velocity to a mean of 13 cm/year (10.3-18) with a mean change of +1.84 ht SDS/year (0.4 to 2.7). This preliminary report suggests the potential efficacy of IDd regimen in promising growth velocity, either directly from a higher dialysis dose or indirectly through an improved nutritional status.

Daily on-line haemodiafiltration: a pilot trial in children.

BACKGROUND: Despite major improvements in paediatric dialysis over the last two decades, cardiovascular outcome is often poor. As France gives priority to kidney transplantation over dialysis, children in chronic haemodialysis are generally pre-adolescents or adolescents with long medical histories and low compliance. In them, the usual weekly schedule of dialysis is often unsuitable. We conducted a study of conversion to daily dialysis, which allowed an enhanced dialysis dose, a gentle ultrafiltration rate and achievement of dry body weight. METHODS: In this single-centre, observational, prospective, non-randomized study, five oligoanuric dialysis patients (mean age: 13.8 +/- 3.2 years) were converted from standard on-line haemodiafiltration (S-OL-HDF) (4 h, three times/week) to daily on-line haemodiafiltration (D-OL-HDF) (3 h, six times/week). Patient selection was based on both the presence of uraemic cardiomyopathy (left ventricular hypertrophy and reduced fractional shortening) and their reduced therapeutic compliance. The D-OL-HDF parameters were the same as for the S-OL-HDF. RESULTS: Increasing the number of sessions from three to six weekly positively impacted the weekly dialysis dose. On D-OL-HDF, mean arterial blood pressure decreased significantly (from 95 +/- 15 to 82 +/- 13 and 87 +/- 9 mmHg at 6 and 12 months, respectively). Left ventricular hypertrophy decreased and its fractional shortening improved markedly (from 26.6 +/- 17% to 31 +/- 14% and 46.6 +/- 15% at 6 and 12 months, respectively). Pre-dialytic plasma phosphorus also decreased markedly (from 1.87 +/- 0.23 to 1.43 +/- 0.22 and 1.28 +/- 0.29 mmol/l at 6 and 12 months, respectively), as did the calcium-phosphorus product. The post-dialytic recovery time disappeared and so did perception of fatigue. Fasting the day before dialysis to avoid excess weight gain (necessitating longer dialysis) disappeared. Combined with an improved appetite, these changes resulted in higher caloric and protein intake (nPCR), from 1.28 +/- 0.23 to 1.43 +/- 0.24 g/kg at 6 months, and school attendance became regular. The only pre-pubertal child included showed catch-up growth. CONCLUSIONS: Increasing dialysis frequency to daily sessions without shortening the durations of sessions excessively allowed us to overcome the "free diet" imposed on these paediatric, very uncompliant patients. This strategy led to a reduction in blood pressure and an improvement of left ventricular size and function, normalization of pre-dialytic plasma phosphorus and improvements in general well-being and dialysis acceptance. Long-term, however, this protocol is only acceptable for the children if associated with the potential of clinical recovery allowing inscription on the kidney transplantation waiting list.

Intraperitoneal pressure in children: fill-volume related and impacted by body mass index.

OBJECTIVE: Prescription of intraperitoneal volume (IPV) in children on peritoneal dialysis (PD) should be individualized in order to optimize both efficiency and tolerance. Intraperitoneal pressure (IPP) can be used as an objective assessment of IPV. Despite IPP being correlated with IPV, there is an important interindividual variability presumed to be secondary to other implicated factors. DESIGN: Over the past 2 years, we conducted a prospective study in our patients on PD (N = 17). For each child, we recorded, at every third month of follow-up, IPV (in milliliters per square meter body surface area), age, gender, body mass index (BMI), and IPP (measured as centimeters of water), collecting a total of 53 data points. RESULTS: There was a positive correlation between IPV and IPP (r = 0.36), but this correlation was weak in contrast to the strong correlation noted between BMI and IPP (r = 0.82). Age had a different influence on IPP: in the younger-aged group (< 2 years), IPP decreased with age; in the older-aged group (> 2 years), IPP increased with age. In some infants, we noted a paradoxical result: a low IPV with a high IPP. CONCLUSIONS: The strong correlation noted between IPP and BMI gives a better understanding of the interindividual variability of the previously described unique relationship between IPP and IPV. The more obese the child (i.e., the higher the BMI), the higher is his IPP for a fixed IPV.